DEFECT OF HUMAN GROWTH-HORMONE RECEPTORS IN THE LIVER OF 2 PATIENTS WITH LARON-TYPE DWARFISM

  • 1 January 1984
    • journal article
    • research article
    • Vol. 20  (1) , 8-11
Abstract
Liver microsome pellets prepared from tissue obtained by elective open biopsy from 2 patients (aged 4 and 26 yr) with Laron-type dwarfism (LTD) showed no specific binding of 125I-hGH (human growth hormone). In contrast, 31 assays of liver microsomes obtained immediately after clinical death from 6 healthy subjects (kidney transplantation donors with a mean age of 24 yr) showed a mean specific binding of 14% (range 7.9-24%). In patients with LTD evidently there is a defect in the hGH receptors of the liver and a consequent lack of somatomedin generation. The fact that the liver microsomes from the 2 LTD patients showed active specific binding of 125I-insulin suggests that, in this syndrome, it is only the receptors for hGH that are defective.